Cystic fibrosis lung disease: Current perspectives
Allergy, Asthma & Respiratory Disease
;
: 3-8, 2020.
Artigo
em Coreano
| WPRIM
| ID: wpr-785354
ABSTRACT
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). These mutations alter the synthesis, processing, function, or half-life of CFTR, the main chloride channel expressed in the apical membrane of epithelial cells in the airway, intestine, pancreas, and reproductive tract. Lung disease is the most critical manifestation of CF. It is characterized by airway obstruction, infection, and inflammation that lead to fatal tissue destruction, which causes most CF morbidity and mortality. This article reviews the pathophysiology of CF, recent animal models, and current treatment of CF.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Pâncreas
/
Mortalidade
/
Canais de Cloreto
/
Regulador de Condutância Transmembrana em Fibrose Cística
/
Modelos Animais
/
Fibrose Cística
/
Obstrução das Vias Respiratórias
/
Células Epiteliais
/
Canais Epiteliais de Sódio
/
Meia-Vida
Tipo de estudo:
Estudo prognóstico
Idioma:
Coreano
Revista:
Allergy, Asthma & Respiratory Disease
Ano de publicação:
2020
Tipo de documento:
Artigo
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